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SUMMARY We report a case of Alexander's disease in a black South African boy of 9 years. The child presented with a grossly abnormal stooped posture, generalised weakness, and slurred nasal speech. Computerised tomography revealed diffuse low radio-density confined to the white matter, and enlarged ventricles. Cerebral biopsy confirmed the diagnosis. The histopathological and electron microscopic features are essentially the same as those described in previous reports. We note the presence of dense clumps of material within some of the Rosenthal fibres, and can find no reference in the literature describing this. Sural nerve biopsy was normal.Case report A black South African boy child aged 9 years and 5 months was first seen at Baragwanath Hospital in July 1978. A rather inadequate history was supplied by his sister. His main complaints were difficulty in walking because of painful knees and backache, and generalised weakness for approximately one month. The patient has six healthy siblings, and his own birth and milestones were apparently normal. From the clinical appearance of the child we considered that he had been ill for longer than the history indicated.He was a thin black male child with a head circumference of 570 mm. He had a strikingly abnormal stooped posture, with flexion of the neck, drooping of the shoulders, and flexion of both hip and knee joints (Fig. 1)

The temporal and spatial pattern of left ventricular (LV) contraction and relaxation in 34 patients with isolated lesions of the left anterior descending artery (LAD) were studied from the LV cineangiogram in the right anterior oblique position using a computer interactive technique. The LV outline was divided into 100 equiangular radii from the center of the enddiastolic silhouette and the trajectory of contraction and relaxation of each radius measured and displayed. Patients were divided into different subsets. Classical anterior wall infarction caused impaired contraction of the distal two thirds of the anterior wall, the apex and the distal quarter of the inferior wall, with marked delay in contraction and relaxation of the border zones and hyperkinesis of the inferior wall. Spatial and temporal disturbances (akinesis and asynchrony) were marked in the other subsets. Left bundle branch block caused profound temporal delay particularly during relaxation.

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